A 10-year-old Chinese girl with two accessory maxillae and bilateral Tessier no. 7 clefts is presented. Radiographic examination showed two accessory maxillae, each containing 5 or 6 supernumerary permanent teeth. The two accessory maxillae extended from the inside of the zygomatic arch to the maxillary tuberosity symmetrically. Duplication of the maxilla is always associated with Amniotic Band Syndrome (ABS), but this case may be a distinct syndrome representing an under-recognised phenotype with bilateral maxillae duplication and Tessier no. 7 clefts.